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Disease Profile
Lichen planopilaris
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Unknown
Age of onset
Adult
ICD-10
L66.1
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
Follicular lichen planus; Frontal fibrosing alopecia (subtype); Kossard disease;
Categories
Skin Diseases
Summary
Lichen planopilaris (LPP) affects the scalp and hair. It is a form of lichen planus, an inflammatory condition affecting the skin and mucous membranes. Symptoms may include scaly skin and redness around hair follicles, bald patches, and pain, burning, or itching on the scalp. Tiny, red bumps (papules) may appear around hair clusters. LLP can cause scarring which leads to permanent hair loss (cicatricial alopecia). There are 3 forms of LPP which differ by the pattern and location of symptoms: classic LPP, frontal fibrosing alopecia, and Lassueur Graham-Little Piccardi syndrome. The cause of LPP is unknown. It is thought to be an auto-immune disorder of the hair follicles. A diagnosis of LPP is made based on a clinical exam and microscopic examination of a piece of
Symptoms
- Patches of hair loss
- Scalp redness
- Scalp pain or burning
- Scalp itching
Lichen planopilaris usually occurs in adulthood between the ages of 30-60. It affects females more than males. It may occur gradually or suddenly, and it gets worse over time. LPP results in scarred patches on the scalp and permanent hair loss.[1]
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
Medical Terms | Other Names |
Learn More:
HPO ID
|
---|---|---|
80%-99% of people have these symptoms | ||
Alopecia |
Hair loss
|
0001596 |
Hyperkeratosis | 0000962 | |
Lichenification | 0100725 | |
Papule | 0200034 | |
30%-79% of people have these symptoms | ||
Abnormal fingernail morphology |
Abnormal fingernails
Abnormality of the fingernails
[ more ] |
0001231 |
Dermal atrophy |
Skin degeneration
|
0004334 |
Pruritus |
Itching
Itchy skin
Skin itching
[ more ] |
0000989 |
Skin ulcer |
Open skin sore
|
0200042 |
5%-29% of people have these symptoms | ||
Abnormal blistering of the skin |
Blistering, generalized
Blisters
[ more ] |
0008066 |
Abnormal intestine morphology |
Abnormality of the intestine
|
0002242 |
Hepatitis |
Liver inflammation
|
0012115 |
Hypopigmented skin patches |
Patchy loss of skin color
|
0001053 |
0100649 | ||
Onycholysis |
Detachment of nail
|
0001806 |
Pterygium | 0001059 |
Diagnosis
Treatment
One specialist who may be involved in the care of someone with lichen planopilaris is a
Related diseases
Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.
Conditions with similar signs and symptoms from Orphanet
|
---|
Differential diagnosis includes discoid lupus erythematosus (DLE), folliculitis decalvans, mucous membrane pemphigoid (see these terms), seborrheic dermatitis, alopecia areata, and central centrifugal cicatricial alopecia (CCCA).
Visit the Orphanet disease page for more information.
|
Organizations
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
Organizations Supporting this Disease
-
Cicatricial Alopecia Research Foundation (C.A.R.F.)
1586 Sumneytown Pike
PO Box 1322
Kulpsville, PA 19443
Telephone: +1-267-613-9811
E-mail: [email protected]
Website: https://www.carfintl.org
Social Networking Websites
- Visit the following Facebook groups related to Lichen planopilaris:
LPP Let's Put Out The Fire
Organizations Providing General Support
-
American Autoimmune Related Diseases Association (AARDA)
19176 Hall Road, Suite 130
Clinton Township, MI 48038
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: [email protected]
Website: https://www.aarda.org/ -
American Hair Research Society (AHRS)
1932 South Halsted Street
Suite 413
Chicago, IL 60608
Telephone: +1-630-578-3991
E-mail: [email protected]
Website: https://www.americanhairresearchsociety.org/
Learn more
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
Where to Start
- DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
- The American Osteopathic College of Dermatology (AOCD) has developed an information page on this condition. Click on the link to read more.
In-Depth Information
- Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Lichen planopilaris. Click on the link to view a sample search on this topic.
References
- Lepe K, Salazar FJ. Lichen Planopilaris. In: StatPearls. Jan 2020; https://pubmed.ncbi.nlm.nih.gov/29262050.
- Babahosseini H, Tavakolpour S, Mahmoudi H, et al. Lichen planopilaris: retrospective study on the characteristics and treatment of 291 patients. J Dermatolog Treat.. 2019; 30(6):598-604. https://pubmed.ncbi.nlm.nih.gov/30411987.
- Kerkemeyer KL, Green J. Lichen planopilaris: A retrospective study of 32 cases in an Australian tertiary referral hair clinic. Australas J Dermatol. 2018; 59(4):297-301. https://pubmed.ncbi.nlm.nih.gov/29297588.
- Errichetti E, Figini M, Croatto M, Stinco G. Therapeutic management of classic lichen planopilaris: a systematic review. Clin Cosmet Investig Dermatol. 2018; 11:91-102. https://pubmed.ncbi.nlm.nih.gov/29520159.
- Randolph MJ, Salhi WA, Tosti A. Lichen Planopilaris and Low-Level Light Therapy: Four Case Reports and Review of the Literature About Low-Level Light Therapy and Lichenoid Dermatosis. Dermatol Ther (Heidelb). 2020; 10(2):311-319. https://pubmed.ncbi.nlm.nih.gov/32060796.
- Meinhard J, Stroux A, Lünnemann L, Vogt A, Blume-Peytavi U. Lichen planopilaris: Epidemiology and prevalence of subtypes a retrospective analysis in 104 patients. J Dtsch Dermatol Ges. 2014 Mar; 12(3):229-35, 229-36. https://www.ncbi.nlm.nih.gov/pubmed/24533855.
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